Review of spectral domain-enhanced depth imaging optical coherence tomography of tumors of the retina and retinal pigment epithelium in children and adults

Abstract

Background:Spectral domain (SD) enhanced depth imaging optical coherence tomography (EDI-OCT) is a useful tool for anatomic, cross-sectional imaging of retinal conditions.Aims:The aim was to identify characteristic patterns of retinal and retinal pigment epithelial tumors on EDI-OCT in children and adults.Settings and Design:Retrospective review.Materials and Methods:Analysis of published reports and personal observations using office-based EDI-OCT for adults and portable hand-held SD OCT for infants and children.Results:Using EDI-OCT, retinal tumors such as small retinoblastoma, astrocytic hamartoma, and hemangioblastoma arose abruptly from the retina, immediately adjacent to normal retina. Small exophytic retinoblastoma and retinal hemangioblastoma showed the full-thickness, homogeneous retinal disorganization with surrounding normal retina “draping” over the margins. Retinoblastoma occasionally had intralesional cavities and surrounding subretinal fluid. Hemangioblastoma often had adjacent intraretinal edema and subretinal fluid. Astrocytic hamartoma arose within the nerve fiber layer and sometimes with a “moth-eaten” or cavitary appearance. Retinal pigment epithelial (RPE) lesions such as congenital hypertrophy of RPE appeared flat with shadowing, occasional subretinal cleft, and abrupt photoreceptor loss. Congenital simple hamartoma showed an abrupt elevation from the inner retina with crisp, dark posterior shadowing. Combined hamartoma of the retina/RPE showed vitreoretinal traction causing “sawtooth mini-peak” or gently “maxi-peak” folding of the retina. RPE adenoma often produces remote macular edema or epiretinal membrane and the tumor has an irregular, “rugged” surface with deep shadowing.Conclusions:Enhanced depth imaging optical coherence tomography shows characteristic patterns that are suggestive of certain retinal and RPE tumors.

Detection and Intraretinal Localization of an ‘Invisible' Retinoblastoma Using Optical Coherence Tomography

“…Several small tumors detected by OCT were reported as being centered in the INL with extension to other layers [2,6], whereas larger tumors have full thickness involvement of the retina or an exophytic mass with relatively normal retina overlying the lesions [6,8,12]. Other small lesions, however, were reported as having ‘outer retinal' involvement with inner ‘retinal draping' over an otherwise smooth, round, homogenous mass [2,12]. To date, only one early lesion detected by HHSD OCT, described by Saktanasate et al [11], appears to be limited to a single retinal layer, in particular the ONL, with the OPL draped over it.…”

“…As OCT has evolved, greater resolution and depth of imaging have led to new applications within ocular oncology [2,3,4]. Handheld spectral-domain OCT (HHSD OCT) allows for imaging in the supine position [5] and therefore is becoming a useful tool in the care of children with retinoblastoma [6].…”

Solitary Circumscribed “Pearl White” Retinal Mass (So-Called Retinal Astrocytic Proliferation) Resides in Deep Retina or Beneath Retina: Findings on Multimodal Imaging in 4 Cases

“…The SD-OCT of this condition closely resembles that of congenital simple hamartoma of the RPE with abrupt vertical margins showing derby hat configuration and dramatic posterior shadowing. 10,11,13 However, congenital simple hamartoma of the RPE is dark black and classically located in the foveal region. The SD-OCT features of solitary circumscribed retinal astrocytic proliferation suggest an origin within the deep retina or RPE.…”

“…The SD-OCT findings of solitary circumscribed retinal astrocytic proliferation are different [8][9][10][11][12][13] than the features of retinal astrocytic hamartoma, which arises in the nerve fiber layer with gentle sloping configuration, and occasionally demonstrate cavitation or "moth-eaten" calcific nodules. 8,9,11,12 The clinical and angiographic features of this condition differ from retinal astrocytoma, which usually manifests a grayyellow color with internal vascularity and retinal exudation.…”

Atypical Retinal Pigment Epithelial Hyperplasia and Glial Proliferation Masquerading as Progressive Recurrent Retinoblastoma: A Case Report Review and Clinicopathologic Correlation

“…Indeed, laser photocoagulation, a common method used for the consolidative treatment of retinoblastoma, has been proven to induce RPE hyperplasia in and around the laser site on the retina using a mouse model [7,8]. However, most pigmentation patterns of RPE hyperplasia and/or hypertrophy are in contrast to retinoblastoma [3]; RPE changes often appear flat and darkly pigmented, occasionally presenting with a subretinal cleft, and do not tend to show significant or rapid growth, thus differentiating RPE hyperplasia from intraocular tumors [9]. Rarely, however, RPE hyperplasia may mimic an intraocular tumor [3,10].…”